Thymoma pathophysiology: Difference between revisions

	Thymoma Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Thymoma from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Thymoma pathophysiology On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Thymoma pathophysiology All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Thymoma pathophysiology
CDC on Thymoma pathophysiology
Thymoma pathophysiology in the news
Blogs on Thymoma pathophysiology
Directions to Hospitals Treating Thymoma
Risk calculators and risk factors for Thymoma pathophysiology

VisualWikitext

Latest revision as of 01:16, 16 August 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amr Marawan, M.D. [2] Ahmad Al Maradni, M.D. [3]Sabawoon Mirwais, M.B.B.S, M.D.[4]

Overview

On gross pathology, a well-circumscribed mass, that is locally invasive, is a characteristic finding of thymoma. On microscopic histopathological analysis, round cells with ample vacuolated cytoplasms and fat droplets are characteristic findings of thymoma.

Pathophysiology

Physiology

Thymus is the site of maturation of T cells.
This makes thymus the primary center responsible for adaptive immunity.

Pathogenesis

The exact pathogenesis of the primary tumor development is not completely understood.

Primary tumors of thymus are relatively rare.
Thymoma is the most common type of primary tumor of thymus.
Thymoma is histologically comprised of abnormally conditioned T cells.
The mingling of these abnormal T cells into the circulation is believed to be involved in the causality of the associated autoimmune disorders.^[1]^[2]

Genetics

Genetic Alterations Reported for the Different WHO Histological Thymoma sub-types^[3]

WHO Type	Chromosomal Gains	Chromosomal Losses
Type A	None	-6p
Type AB	None	-5q21 - 22 -6q -12p -16q
Type B3	+1q	-6 -13q

Associated Conditions

Approximately 30% of the patients have their thymomas discovered because of a symptomatic associated autoimmune disorder. These disorders include:^[4]

Type	Diseases
Neuromuscular diseases	Myasthenia gravis Neuromyotonia Rippling muscle disease Polymyositis/dermatomyositis Encephalitis (limbic, cortical and brain stem) Intestinal pseudoobstruction
Hematologic autoimmune diseases	Anemia: Pure red cell aplasia, pernicious anemia, hemolytic anemia, aplastic anemia. Other isolated cytopenia: Eosinophils, basophils, neutrophils Immunodeficiencies: Hypogammaglobulinaemia, Good syndrome
Dermatologic disorders	Pemphigus (foliaceus or paraneoplastic) Lichen planus Alopecia areata
Endocrine disorders	Addison disease Graves disease Cushing's disease
Renal and hepatic diseases	Glomerulonephritis Autoimmune hepatitis
Systemic autoimmune diseases	SLE Sjögren's syndrome Systemic sclerosis Graft-versus-host disease

Gross Pathology

On gross pathology, a well circumscribed mass, that is locally invasive, is a characteristic finding of thymoma.

Microscopic Pathology

On microscopic histopathological analysis, round cells, with ample vacuolated cytoplasms, and fat droplets are characteristic findings of thymoma.

Video

References

↑ C. Buckley, D. Douek, J. Newsom-Davis, A. Vincent & N. Willcox (2001). "Mature, long-lived CD4+ and CD8+ T cells are generated by the thymoma in myasthenia gravis". Annals of neurology. 50 (1): 64–72. PMID 11456312. Unknown parameter |month= ignored (help)
↑ J. V. Souadjian, P. Enriquez, M. N. Silverstein & J. M. Pepin (1974). "The spectrum of diseases associated with thymoma. Coincidence or syndrome?". Archives of internal medicine. 134 (2): 374–379. PMID 4602050. Unknown parameter |month= ignored (help)
↑ "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). Retrieved 26 February 2014. External link in |title= (help)
↑ "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). External link in |title= (help)

Template:WikiDoc Sources

[1] C. Buckley, D. Douek, J. Newsom-Davis, A. Vincent & N. Willcox (2001). "Mature, long-lived CD4+ and CD8+ T cells are generated by the thymoma in myasthenia gravis". Annals of neurology. 50 (1): 64–72. PMID 11456312. Unknown parameter |month= ignored (help)

[2] J. V. Souadjian, P. Enriquez, M. N. Silverstein & J. M. Pepin (1974). "The spectrum of diseases associated with thymoma. Coincidence or syndrome?". Archives of internal medicine. 134 (2): 374–379. PMID 4602050. Unknown parameter |month= ignored (help)

[3] "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). Retrieved 26 February 2014. External link in |title= (help)

[4] "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). External link in |title= (help)

[1]

[2]

[3]

[4]

@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Thymoma}}
-{{CMG}}; {{AE}} {{AM}} {{AAM}}
+{{CMG}}; {{AE}} {{AM}} {{AAM}}{{Sab}}
 ==Overview==
-* On gross pathology, a well circumscribed mass that is locally invasive is a characteristic finding of thymoma.
+On [[gross pathology]], a well-circumscribed mass, that is locally [[Invasive (medical)|invasive]], is a characteristic finding of thymoma. On [[microscopic]] [[Histopathology|histopathological]] [[analysis]], round [[Cell (biology)|cells]] with ample vacuolated [[Cytoplasm|cytoplasms]] and [[fat]] droplets are characteristic findings of thymoma.
-* On microscopic histopathological analysis, round cells, with ample vacuolated cytoplasms, and fat droplets are characteristic findings of thymoma.
 ==Pathophysiology==
-===Origin===
-*It has been believed that thymic epithelium is derived from both [[ectoderm]]al and [[endoderm]]al stem cells.
-*Recent evidence indicates that epithelial populations originate from a common progenitor of endodermal origin.
-*Occurrence of more differentiated “committed stem cells” with medullary, cortical or other phenotypes is possible.<ref name="Blackburn-2002">{{Cite journal  | last1 = Blackburn | first1 = CC. | last2 = Manley | first2 = NR. | last3 = Palmer | first3 = DB. | last4 = Boyd | first4 = RL. | last5 = Anderson | first5 = G. | last6 = Ritter | first6 = MA. | title = One for all and all for one: thymic epithelial stem cells and regeneration. | journal = Trends Immunol | volume = 23 | issue = 8 | pages = 391-5 | month = Aug | year = 2002 | doi =  | PMID = 12133801 }}</ref><ref name="Gill-2002">{{Cite journal  | last1 = Gill | first1 = J. | last2 = Malin | first2 = M. | last3 = Holländer | first3 = GA. | last4 = Boyd | first4 = R. | title = Generation of a complete thymic microenvironment by MTS24(+) thymic epithelial cells. | journal = Nat Immunol | volume = 3 | issue = 7 | pages = 635-42 | month = Jul | year = 2002 | doi = 10.1038/ni812 | PMID = 12068292 }}</ref>
-===Microscopic Pathology===
+=== Physiology ===
-On microscopic histopathological analysis, round cells, with ample vacuolated cytoplasms, and fat droplets are characteristic findings of thymoma.
-===Gross Pathology===
+*[[Thymus]] is the site of maturation of [[T cell|T cells]].
-On gross pathology, a well circumscribed mass that is locally invasive is a characteristic finding of thymoma.
+* This makes [[thymus]] the primary center responsible for [[adaptive immunity]].
-{|
+=== Pathogenesis ===
-| [[File:T007.png|x300px|thumb|Mediastinum: locally invasive, circumscribed [[Thymoma]]. Its cut surface bulges, and is pale tan and faintly lobulated. It invaded the capsule at a few points but still remained within the thymus.]]
-|}
+* The exact [[pathogenesis]] of the [[primary tumor]] development is not completely understood.
+*[[Primary tumor|Primary tumors]] of [[thymus]] are relatively rare.
+* Thymoma is the most common type of [[primary tumor]] of [[thymus]].
+* Thymoma is [[Histology|histologically]] comprised of abnormally conditioned [[T cell|T cells]].
+* The mingling of these abnormal [[T cell|T cells]] into the [[Circulatory system|circulation]] is believed to be involved in the [[causality]] of the associated [[Autoimmunity|autoimmune disorders]].<ref>{{Cite journal
+ | author = [[C. Buckley]], [[D. Douek]], [[J. Newsom-Davis]], [[A. Vincent]] & [[N. Willcox]]
+ | title = Mature, long-lived CD4+ and CD8+ T cells are generated by the thymoma in myasthenia gravis
+ | journal = [[Annals of neurology]]
+ | volume = 50
+ | issue = 1
+ | pages = 64–72
+ | year = 2001
+ | month = July
+ | pmid = 11456312
+}}</ref><ref>{{Cite journal
+ | author = [[J. V. Souadjian]], [[P. Enriquez]], [[M. N. Silverstein]] & [[J. M. Pepin]]
+ | title = The spectrum of diseases associated with thymoma. Coincidence or syndrome?
+ | journal = [[Archives of internal medicine]]
+ | volume = 134
+ | issue = 2
+ | pages = 374–379
+ | year = 1974
+ | month = August
+ | pmid = 4602050
+}}</ref>
-===Genetic Features===
+==Genetics==
-'''Genetic alterations reported for the different WHO histological thymomasubtypes'''<ref>{{Cite web  | last =  | first =  | title = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | url = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | publisher =  | date =  | accessdate = 26 February 2014 }}</ref>
+'''Genetic Alterations Reported for the Different WHO Histological Thymoma sub-types'''<ref>{{Cite web  | last =  | first =  | title = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | url = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | publisher =  | date =  | accessdate = 26 February 2014 }}</ref>
 {| border="1" cellpadding="2"
 |-
@@ Line 31: / Line 50: @@
 | width="200pt" |'''Chromosomal Losses'''
 |-
-|Type A || none || -6p
+!Type A
+|
+* None
+|
+* -6p
 |-
-|Type AB || none || -5q21-22,-6q,-12p,-16q
+!Type AB
+|
+* None
+|
+* -5q21 - 22
+* -6q
+* -12p
+* -16q
 |-
-|Type B3 || +1q || -6,-13q
+!Type B3
+|
+* +1q
+|
+* -6
+* -13q
 |}
-{|
+==Associated Conditions==
+Approximately 30% of the [[Patient|patients]] have their thymomas discovered because of a symptomatic associated [[autoimmune disorder]]. These disorders include:<ref>{{Cite web  | last =  | first =  | title = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | url = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | publisher =  | date =  | accessdate = }}</ref>
-==Video==
-{{#ev:youtube|wfyixp6JxQM}}
-==Associated Disorders==
-Approximately 30% of patients have their thymomas discovered because they have an associated autoimmune disorder. These disorders include:<ref>{{Cite web  | last =  | first =  | title = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | url = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | publisher =  | date =  | accessdate = }}</ref>
 {| {{table}} cellpadding="4" cellspacing="0" style="border:#c9c9c9 1px solid; margin: 1em 1em 1em 0; border-collapse: collapse;"
 | style="width: 25%;" | '''Type'''
 | style="width: 75%;" | '''Diseases'''
 |-
-| Neuromuscular Diseases
+![[Neuromuscular disease|Neuromuscular diseases]]
-|[[Myasthenia gravis]], [[neuromyotonia]], rippling muscle disease, [[polymyositis]]/[[dermatomyositis]], [[encephalitis]] (limbic, cortical and brain stem), [[intestinal pseudoobstruction]]
+|
+* [[Myasthenia gravis]]
+* [[Neuromyotonia]]
+* Rippling [[muscle]] [[disease]]
+* [[Polymyositis]]/[[dermatomyositis]]
+* [[Encephalitis]] ([[Limbic encephalitis|limbic]], cortical and [[brain stem]])
+* [[Intestinal pseudoobstruction]]
 |-
-| Haematologic Autoimmune Diseases
+![[Hematology|Hematologic]] [[autoimmune diseases]]
-|[[Anemia]]: [[pure red cell aplasia]], [[pernicious anemia]], [[hemolytic anemia]], [[aplastic anemia]]. Other isolated cytopenia: [[eosinophils]],[[basophils]], [[neutrophils]], immunodeficiencies: [[hypogammaglobulinaemia]] +/- T-cell deficiencies ([[Good syndrome]])
+|
+* [[Anemia]]: [[Pure red cell aplasia]], [[pernicious anemia]], [[hemolytic anemia]], [[aplastic anemia]].
+* Other isolated [[cytopenia]]: [[Eosinophils]], [[basophils]], [[neutrophils]]
+* [[Immunodeficiency|Immunodeficiencies]]: [[Hypogammaglobulinaemia]], [[Good syndrome]]
 |-
-| Dermatologies Diseases
+![[Dermatologic disorders]]
-|[[Pemphigus]] (foliaceus or paraneoplastic), [[lichen planus]], [[alopecia areata]]
+|
+* [[Pemphigus]] (foliaceus or [[paraneoplastic]])
+* [[Lichen planus]]
+* [[Alopecia areata]]
 |-
-| Endocrine Disorders
+![[Endocrine disorders]]
-|[[Addison disease]], [[graves disease]], [[Cushing's disease]]
+|
+* [[Addison disease]]
+* [[Graves disease]]
+* [[Cushing's disease]]
 |-
-| Renal and Hepatic Diseases
+![[Kidney|Renal]] and [[Liver|hepatic]] [[Disease|diseases]]
-|[[Glomerulonephritis]], [[autoimmune hepatitis]]
+|
+* [[Glomerulonephritis]]
+* [[Autoimmune hepatitis]]
 |-
-| Systemic Autoimmune Diseases
+![[Systemic]] [[autoimmune diseases]]
-|[[SLE]], [[Sjögren's syndrome]], [[systemic sclerosis]], [[graft-versus-host disease]]
+|
+* [[SLE]]
+* [[Sjögren's syndrome]]
+* [[Systemic sclerosis]]
+* [[Graft-versus-host disease]]
 |}
+==Gross Pathology==
+On [[gross pathology]], a well circumscribed mass, that is locally [[Invasive (medical)|invasive]], is a characteristic finding of thymoma.
+==Microscopic Pathology==
+On [[microscopic]] [[histopathological]] [[analysis]], round [[Cell (biology)|cells]], with ample vacuolated [[Cytoplasm|cytoplasms]], and [[fat]] droplets are characteristic findings of thymoma.
+{|
+==Video==
+{{#ev:youtube|wfyixp6JxQM}}
 ==References==